EDS Skin vs Normal Skin, A-Z of Ehlers-Danlos Syndrome

adminSeptember 9, 2022

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How different is EDS skin vs normal skin. Patients with EDS (Ehlers-Danlos Syndrome) have different skin and joint characteristics than others. This is because the condition affects connective tissues which could be found in many places including the skin.

EDS skin is more elastic than normal skin and might need more maintenance, especially when there is an injury.

Read on to learn about Ehlers-Danlos Syndrome, its causes, and how it affects the skin.

What Is Ehlers-Danlos Syndrome (EDS)?

Ehlers-Danlos Syndrome is used to address a group of genetic disorders that affect connective tissues. Connective tissues are substances made up of a mixture of proteins. These substances maintain the framework, strength, and elasticity of body structures.

Connective tissues are primarily found in the skin, joints, and blood vessels. They could also be seen in the bed of viscera structures inside the body.

The common symptoms of this condition include;

Hypermobile joints; are joints that exceed the normal range of movement.
Elastic or hyperextensible skin
Injury-prone skin
Rupture of arteries, intestines, or urinary tract
Scoliosis
Poor muscle tone
Severe gum disease

These symptoms are not seen in all types of EDS as different genes are affected in the different types.

Types of EDS skin vs normal skin

There are 13 types of Ehlers-Danlos Syndrome (EDS) which are;

#1.Classical EDS

Generally, this type of EDS is characterized by generalized joint hypermobility and elastic skin with atrophic scarring.

#2.Classical-like EDS (clEDS)

The common features of this type of EDS are joint hypermobility, skin hyperextensibility with a velvety surface, and easily bruised skin.

#3.Hypermobile EDS

This type of EDS does not have any molecular or genetic cause (as of now). Therefore diagnoses are done by clinical hypothesis.

#4.Vascular EDS (vEDS)

This type of EDS is common and is characterized by; arterial rupture, perforation of the sigmoid colon, uterine rupture, and formation of carotid-cavernous sinus fistula.

#5.Cardiac-vascular EDS (cvEDS)

As the name, the common characteristic of this type of EDS is problems with the aortic and mitral valves, joint hypermobility, and several skin issues.

#6.Arthrochalasia EDS (arEDS)

This EDS is identified by skin hyperextensibility, congenital and bilateral hip dislocation, and severe GJH with dislocation and subluxations.

#7.Dermatosparaxis EDS (dEDS)

This type of EDS has various minor and major criteria including skin fragility and craniofacial features.

#8.Kyphoscoliotic EDS (kEDS)

This type of EDS has these characteristics; congenital muscle hypotonia, kyphoscoliosis, and severe general joint hypermobility associated with joint dislocations and subluxations.

$9.Brittle cornea syndrome (BCS)

The features seen in this type of EDS include; brittle cornea that might or might not rupture, early and progressive keratoconus, and keratoglobus.

#10.Spondylodysplastic EDS (spEDS)

These characteristics are seen in this type of EDS; Bow limbs, dwarfism, and muscle hypotonia.

#11.Musculocontractual EDS (mcEDS)

In this type of EDS, there are multiple joint contractures, craniofacial issues, and uncommon skin features like hyperextensibility.

#12. Myopathic EDS (mEDS)

This type of EDS is associated with muscle hypotonia and atrophy, hypermobility of distal joints, and contracture of proximal joints.

#13.Periodontal EDS (pEDS)

This type of characterized by severe periodontitis in early childhood or adolescent age, poorly attached gingiva, pretibial plaques, etc.

Causes Of Ehlers-Danlos Syndrome-EDS Skin vs Normal Skin

In the body, there are different types of important tissues, and connective tissue is one of them. Connective tissues give support, structure, and elasticity to structures in the body. several factors have been attributed for the differences between EDS skin vs normal skin.

Connective tissues are made up of several proteins, one of which is collagen. In diseases like Ehler-Danlos Syndrome, the genes that make collagen are faulty. The different types of EDS are caused by different genes of collagen production that are faulty.

That being said, these faulty genes of collagen could be inherited via two patterns; autosomal dominant and autosomal recessive patterns. Sometimes, these genes might not be inherited and would happen for the first time in an individual.

How Does EDS Affect The Skin?

Most types of EDS affect the basic characteristics of the skin as it contains plenty of connective tissues. The most common effect is hyperextensibility of the skin.

In almost all types of EDS, especially classical EDS, this skin feature is very prominent. It is, however, absent in vascular EDS where the skin is thin and transparent-like and blood vessels are very visible.

Even though hyperextensibility is the most common EDS skin feature, different types of EDS give different skin features.

Features Of EDS skin vs normal skin

Different features distinguish the EDS skin vs normal skin , among these are the following predominant features.

#1 Soft Skin

People with Ehler-Danlos Syndrome (EDS) have abnormal soft skin on certain sites. This is commonly seen in specific areas of the body -especially the forearms.

$2 Fragile skin

EDS skin is prone to tears, cuts, and bruises from slight fiction or normal activities that might not cause a tear on normal skin. Children with this condition, are prone to falling easily due to unstable joints.

Being fragile, the skin is prone to thin atrophic scars (not severe scars).

#3 Hyperextensibility

People with EDS have elastic skin, that is, skin that can be stretched beyond the normal range, and recoils back to its initial position.

You could do a skin test by gently pulling a portion of skin on the hairless part of the forearm. Pull the skin until it reaches its limit. If the skin was stretched by more than 1.5cm, it is hyperextensible. Do not do this test on skin that is over a joint, as you would get the wrong results.

#4 Haematomas

Due to fragile blood capillaries, and weak structure of the skin, people with EDS are prone to easily obtain bruises from little trauma. Therefore surplus bleeding from minor injuries is not a clotting abnormality, but a skin abnormality in the case of EDS.

#5 Piezogenic papules

These are small lumps that appear on the sides of the heel when standing and might disappear when the foot is lifted. They are less than 1cm in length, soft, skin-colored, and might be painful.

Piezogenic papules are a minor feature of EDS skin as they might not be seen in most cases. It could also be found in people that do not have EDS.

#5 Meischer’s elastoma

This feature of EDS skin is characterized by a circular pattern that has rough edges and a clear center. It is mostly seen on the neck and other flexures, like the elbow, knee, etc.

It is caused by broken elastic fibers that protrude from the dermis into the epidermis. This feature is also found in other collective tissue conditions like Marfan’s syndrome, pseudoxanthoma elasticum, and osteogenesis imperfecta.

#6 Little or no response to local anesthetic

It was proven in 1990, that patients of EDS have little or no response to local anesthetic, while they respond perfectly to general anesthetic. The problem was likely due to the rapid dispersal of the anesthetic in EDS skin than in normal skin.

Summary

EDS skin vs normal skin, is a topical issue.

Ehler-Danlos Syndrome (EDS) is an inherited disorder that is characterized by faulty genes of collagen which is a major component of connective tissues. Connective tissues are found in many places including the skin.

This makes EDS skin different from normal skin with certain features mentioned above.

adminSeptember 9, 2022

0 16 5 minutes read

EDS Skin vs Normal Skin, A-Z of Ehlers-Danlos Syndrome

What Is Ehlers-Danlos Syndrome (EDS)?